The document describes an arteriovenous malformation (AVM) in a 22-year-old male patient. Key details include: - The patient presented with pain in his right gluteal region and noticed dilated veins. Imaging showed a large AVM in the right gluteal region fed by branches of the right internal iliac artery and draining into the right internal iliac vein. - The patient underwent embolization to reduce blood flow by 95% prior to planned excision of the AVM. - AVMs result from an error in vascular development and cause direct shunting of blood from arteries to veins without an intervening capillary bed. Management options aim to alleviate symptoms

1. ARTERIOVENOUS
MALFORMATIONS
Dr Avijit Banerjee (Surgery
PGT)
Dr AK Malhotra ( CHIEF
SURGEON)
SOUTH EASTERN RAILWAY
HOSPITAL
KOLKATA

2. THE CASE
A 22 year old boy was admitted with history of pain in the right
gluteal region for the last 2 months
Patient noticed dilated veins in right gluteal region
There was no h/o altered bowel habits , or bleeding per rectum
, or hematuria
No history of fever, loss of appetite, loss of weight
The swelling is not associated with any ulceration or satellite
nodules.
No h/o cough, hemoptysis, bone pain, headache, vomiting or
jaundice
No h/o previous excision of the swelling or presence of similar
swelling in the past.

3. INSPECTION
There was a single ill defined swelling about 7x10cm
on the right buttock
There was no associated venous
prominence/ulceration/scar mark/satellite nodule
Cough impulse not seen
No associated pressure symptoms leading to wasting
of muscles or swelling of lower limbs

4. PALPATIONThere is no change of temperature over the
swelling or tenderness.
There is presence of thrill on palpation over the
swelling
The mass in soft in consistency and
compressible
Fluctuation and transillumination test is negative
No palpable cough impulse
Normal movement of adjacent joints
Examination of regional lymph nodes did not
reveal any abnormality
Per rectal examination reveals bulge on the right
side along with thrill

5. RADIOLOGICAL INVESTIGATIONS
USG
ARTERIOVENOUS
MALFORMATION IN THE RIGHT
GLUTEAL MUSCLES
MRI
A LARGE 11.7x10.8x5.9cm AVM
IN RIGHT GLUTEAL REGION
THE LESION IS EXTENDING TO
THE ISCHIORECTAL AND
PERIANAL SPACE WITHOUT ANY
EXTENSION TO THE
INTERSPHINCTERIC REGION
FEEDING FROM BRANCHES OF
RIGHT INTERNAL ILIAC ARTERY
AND DRAINING INTO INTERNAL
ILIAC VEIN

8. ANGIOGRAPHY-A LARGE AVM AT RIGHT
GLUTEAL REGION FEEDING FROM BRANCHES OF
RIGHT INTERNAL ILIAC ARTERY AND DRAINING INTO
RIGHT INTERNAL ILIAC VEIN

9. THE COURSE OF TREATMENT
Embolization of pelvic AVM done
>95% flow reduction achieved
Plan for excision of AVM in right
gluteal region done under general
anaesthesia after taking high risk
consent and consent for colostomy

21. POST OP RESULTS

22. ARTERIOVENOUS MALFORMATION
Arteriovenous malformation (AVM) results from an error of vascular
development between the 4th and 6th weeks of gestation
According to Halsted it results from failure of arteriovenous channels in
the primitive retiform plexus to regress
AVM is 20 times more common in the central nervous system, where
apoptosis is rare
An absent capillary bed causes shunting of blood directly from the arterial
to-venous circulation, through a fistula or nidus (abnormal channels
bridging the feeding artery to the draining veins)

23. Hereditary haemorrhagic telangiectasia
(HHT) results from mutations in endoglin
and activin receptor like kinase 1, which
affect TGF-b signalling
Capillary malformation-arteriovenous
malformation (CM-AVM) is caused by a
mutation in RASA1
AVM may enlarge because of increased
blood flow causing collateralization,
dilatation of vessels (especially venous
ectasia), and thickening of adjacent arteries
and veins
Shunting and aneurysms due to trauma or

24. The most common site of extra cranial AVM is the head and neck,
followed by the limbs, trunk, and viscera
Early lesions present as a pink-red cutaneous stain, without a
palpable thrill or bruit
In future, the patient is at risk for pain, ulceration, and bleeding

25. SCHOBINGER STAGING OF AVM

26. DIAGNOSIS
Most AVMs are diagnosed by history and physical examination
Hand-held Doppler reveals fast-flow and excludes a slow-flow vascular
anomaly
MRI also is necessary to: (1) confirm the diagnosis; (2) determine the extent
of the lesion; (3) plan treatment
If the diagnosis remains unclear after US and MRI, angiography is
sometimes needed
CT may be indicated if the AVM involves bone
Histopathological diagnosis of AVM is rarely necessary, but may be
indicated to rule out malignancy or if imaging is equivocal

27. MANAGEMENT
Intervention is focused on alleviating symptoms (i.e., bleeding, pain,
ulceration), preserving vital functions (i.e., vision, mastication), and
improving deformity as cure is rare as it is generally around important
structures
Management options include embolization, resection, or a combination
Stage 1 and 2 is only where long term control or “cure” can be possible
Stage III and IV AVMs require intervention to control pain, bleeding,
ulceration, or congestive heart failure.

28. EMBOLIZATION
Embolization is used either as a preoperative adjunct to resection or as
monotherapy
Because the AVM is not removed, almost all lesions eventually re-expand
after treatment
Most recurrences occur within the first year after embolization, and 98% re-
expand within 5 years
Substances used for embolization are either liquid (n-butyl cyanoacrylate
(n-BCA), Onyx) or solid (polyvinyl alcohol particles (PVA), coils)
The goal of embolization is occlusion of the nidus and proximal venous
outflow

29. For preoperative embolization, temporary
occlusive substances (Gel foam powder, PVA,
embospheres) that undergo phagocytosis are
used. Permanent liquid agents capable of
permeating the nidus (n-BCA, Onyx) are
employed when embolization is the primary
treatment
The most frequent complication of embolization
is ulceration

30. RESECTION
Wide extirpation and reconstruction of large, diffuse AVM should be
exercised with caution because: (1) cure is rare and the recurrence rate is
high; (2) the resulting deformity is often worse than the appearance of the
malformation; (3) resection is associated with significant blood loss,
iatrogenic injury, and morbidity
Preoperative embolization will facilitate the procedure by reducing the
size of the AVM, minimizing blood loss, and creating scar tissue to aid the
dissection
Excision should be done 24–72 h after embolization, before recanalization
restores blood flow to the lesion

31. RECONSTRUCTION
Skin grafting over ulcerated areas has a high failure
rate because the underlying tissue is ischemic;
excision with regional flap transfer may be required

32. THANK YOU